CASE REPORT

Sonography of multifocal hydatidosis involving lung and liverin a female child

Abhishek Jha • Ekram Ullah • Prakhar Gupta •

Gagan Gupta • Mohd. Saud

Received: 17 December 2012 / Accepted: 27 February 2013 / Published online: 11 April 2013

� The Japan Society of Ultrasonics in Medicine 2013

Abstract Hydatid disease, caused by Echinococcus

granulosus, is a zoonotic infection encountered worldwide.

Though involvement of the liver and lungs is quite com-

mon, pelvic involvement is rarely reported, with the inci-

dence being 0.2–2.2 %. Ovarian and broad ligament

hydatids are rare entities and are usually seen after rupture

of a hepatic hydatid cyst. These cysts are usually asymp-

tomatic, and a high index of clinical suspicion coupled with

unequivocal imaging findings is required to make an

accurate and timely diagnosis. We present a case of mul-

tifocal hydatid disease in a female child involving the lungs

and liver and provide an account of the quintessential

radiological findings.

Keywords Hydatidosis � Lung hydatid � Hepatic hydatid �Double wall � Ultrasound

Introduction

Hydatid disease is a parasitic zoonosis caused by the larval

stage of Echinococcus granulosus and, uncommonly,

Echinococcus multilocularis. Canines are the definite hosts

of Echinococcus, and human beings and cattle are inter-

mediate hosts. Epidemiologically, it is found everywhere in

the world, and is especially common in Asian and African

countries. The most common organ to be involved in

hydatid disease is the liver (60–70 %), followed by the

lungs (10–25 %), while ovarian and parametrial involve-

ment is extremely uncommon [1]. Multiorgan involvement

is exceedingly rare and occurs in less than 2 % of the cases.

Diagnosis is usually established by serological tests and

radiological imaging. The optimal treatment of such cases

includes albendazole followed by surgical excision.

Case report

An 8-year-old female child from a rural background pre-

sented to the pediatric outpatient department with com-

plaints of difficulty in breathing and recurrent abdominal

pain. The patient had no history of any other chronic ill-

ness. On examination, vital signs of the patient were stable.

General examination revealed pallor. On respiratory

examination, there was reduced air entry bilaterally, and

fine crepitations were auscultated in bilateral lung bases.

Abdominal examination revealed tender hepatomegaly.

The rest of the examination was within normal limits.

Preliminary routine investigations revealed raised

eosinophil count (17 %). Chest radiograph of the patient

revealed large, well-defined, spherical radio-opaque shad-

ows, with no air–fluid levels or apparent calcification, in

bilateral mid lung zones (Fig. 1). The medial border of the

right-sided lesion was indistinct from the right cardiac

border. There was no evidence of effusion. The patient was

then subjected to abdominal ultrasound examination, which

revealed hepatomegaly, with multiple multilocular cystic

lesions with multiple internal septations, and no

A. Jha � E. Ullah � G. Gupta � Mohd. Saud

Department of Radiodiagnosis, Jawaharlal Nehru Medical

College, Aligarh Muslim University, Aligarh, India

A. Jha (&)

Defender Locks and Hardware, Achal Tank,

Aligarh 202001, India

e-mail: abhi.jnmc@gmail.com

P. Gupta

Department of General Surgery, Jawaharlal Nehru Medical

College, Aligarh Muslim University, Aligarh, India

123

J Med Ultrasonics (2013) 40:471–474

DOI 10.1007/s10396-013-0444-5

calcifications, distributed in both lobes of the liver (Figs. 2,

3). No free fluid was detected. A provisional diagnosis of

multifocal hydatid disease was made.

Thereafter, the patient underwent computed tomography

(CT) scanning of the thorax and the abdomen to confirm

the diagnosis, ascertain the extent of the lesion, and rule out

other possibilities. Contrast-enhanced CT scan of the tho-

rax revealed well-defined, unilocular, spherical, fluid

attenuation, cystic lesions with minimally enhancing walls

in the right middle lobe and left lower lobe (Fig. 4). There

was no evidence of calcifications within the lesions. No

definite mediastinal lymphadenopathy was noted, and no

apparent communication with bronchi was seen. CT scan

of the abdomen revealed multiple multilocular cystic

lesions, with thick internal septations producing a honey-

comb or cartwheel appearance. These lesions were present

in both hepatic lobes (Fig. 5).

ELISA for echinococcal immunoglobulin E was

49.7 Ku/L. The clinical picture combined with the radio-

pathological appearance strongly favored the diagnosis of

multifocal hydatidosis. The patient was started on alben-

dazole, and after 2 weeks she was taken up for surgery.

A midline incision was made and the surgical field

involving the peritoneal cavity was packed with sponges

dipped in 0.5 % silver nitrate. The largest of the hepatic

cysts was then aspirated through a suction device and

daughter cysts were evacuated. The residual pericystic

cavity was then filled with normal saline and closed by

purse-string sutures. Three more similar cysts were excised

in this fashion. The cyst wall was sent for histopathological

examination, which confirmed the diagnosis of hydatid

cyst. No postoperative complications were seen. No defi-

nite surgical procedure was planned for pulmonary hydatid

cysts, which showed marked regression in size following

medical therapy.

Discussion

Hydatid disease is worldwide in distribution and is espe-

cially common in countries where animal husbandry is

largely practiced. The most common organs to be involved

include the liver and lungs. Many authors report pulmonary

involvement to be the commonest in the pediatric popu-

lation [2, 3]. It is hypothesized that increased compliance

of lung tissue as compared to the liver favors expansion

and growth of these cysts. Pelvic hydatid cysts are rarely

primary and are most commonly seen secondary to inoc-

ulation from rupture of a hepatic hydatid [4–6].

Clinically, these cysts are usually asymptomatic when

small. With growth in size, symptoms secondary to a mass

effect of these cysts arise. Large hepatic hydatid cysts may

cause abdominal pain and obstructive jaundice. Cough and

chest pain are the most common features of pulmonary

hydatids. Symptoms may also arise due to complications

like rupture of cyst, secondary infection, and formation of

cystobiliary fistulas, which may include anaphylaxis, urti-

caria, or cholangitis.

Pathologically, hydatid cysts consist of three layers. The

outermost layer is fibrous and is called pericyst. It is

formed due to inflammatory changes secondary to activa-

tion of the immune system of the host. Pericyst is absent in

hydatid cysts located in brain and lungs. The middle layer

is acellular and is called the laminar membrane or the

ectocyst, and the inner layer is the germinal layer, which is

the vital layer and harbors the parasites, also known as

endocyst [7, 8]. The fluid within the cyst is alkaline and is

highly antigenic. Peritoneal spillage of this fluid can result

in severe anaphylactic reaction.

Imaging plays a pivotal role in the diagnosis of these

lesions. The most common imaging modalities include

ultrasonography, CT scan, and magnetic resonance imaging

Fig. 1 Chest X-ray

posteroanterior and lateral

views showing large, well-

defined, spherical, radio-opaque

shadows, with no air–fluid

levels or apparent calcification,

in bilateral mid lung zones

472 J Med Ultrasonics (2013) 40:471–474

123

(MRI). Plain chest radiograph is a valuable tool in the initial

diagnosis of pulmonary hydatid. Several radiographic signs

of pulmonary hydatid cysts have been described in the lit-

erature, including notch sign, water-lily sign, meniscus sign,

annular solar eclipse, and the migratory sign.

Ultrasonography is an essential tool in the diagnosis of

hydatid cysts. Several sonographic signs have been

described in relation to hydatid cysts. On ultrasonography,

the cyst wall is seen as two echogenic lines separated by

linear hypoechogenicity [9], the so-called ‘‘double wall’’

sign. The motion of fine echogenic debris with change in

the patient’s posture can be well appreciated, and is also

referred to as the ‘‘snow storm’’ sign [9]. Detachment of the

endocyst from the pericyst can result from trauma,

degeneration or host tissue reaction. In such cases, visible

‘‘floating membranes’’ can be appreciated within the cyst.

In some cases, a focal rent in the wall can also be noticed.

When the detachment is complete, the sonographic ‘‘water-

lily sign’’ can be seen, which is so named because of the

resemblance of pulmonary hydatids to waterlily. With

disease progression, the physical properties of the hydatid

fluid also undergo transition from watery to more viscid

fluid, resulting in the folds of the germinal layer becoming

immobile and being seen as serpentine structures entrapped

within an echogenic matrix. This appearance is called the

‘‘congealed water-lily’’ sign. Daughter cysts are visualized

as multiple cysts separated from each other by well-defined

walls and may give rise to a multilocular or honeycomb

appearance of these lesions. In conditions where the cyst

cavity is completely obliterated by the matrix, the cyst may

appear as a solid mass [10]. Calcification, both within the

wall as well as within the matrix, can also be well depicted

by ultrasound. Ultrasound stands out as the most sensitive

Fig. 3 Transabdominal ultrasonography with a 3.75-MHz curvilinear

probe showing multiple small anechoic cysts within a larger cyst,

referred to as ‘‘daughter’’ cysts

Fig. 4 Contrast-enhanced CT scan of the patient shows cystic lesions

with well-defined spherical fluid intensity and high attenuation wall in

the right middle lobe in the paracardiac location and the left lower

lobe, with no evidence of calcification, air–fluid level, or daughter

cysts

Fig. 2 a Transabdominal ultrasonography with a 3.75-MHz curvi-

linear probe showing well-defined multilocular cystic lesions in the

right hepatic lobe giving rise to a honeycomb appearance and b the

same lesion with a linear 7.5-MHz probe showing the ‘‘double wall’’

of these cystic lesions. Note the outer wall (straight white arrow)

representing the pericyst and the inner layer (curved white arrow)

representing the ectocyst. No definite evidence of calcifications is

seen

J Med Ultrasonics (2013) 40:471–474 473

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imaging modality in detection of septa, membranes, and

hydatid sand [9].

CT scan clearly depicts most of the findings that are

seen on sonography. On contrast-enhanced CT scan, these

cysts show a high attenuation wall. Calcifications, detached

membranes, and daughter cysts can be easily seen on CT

scan. CT scan is also the investigation of choice in detec-

tion of cyst infection. On MRI, these cysts contain a hyp-

ointense peripheral rim on T2W sequences, which is

represented by pericyst, which is rich in collagen [11]. The

daughter cysts are hypointense on T1W sequences and

hyperintense on T2W sequences in comparison with cyst

fluid. Ovarian and broad ligament hydatids are extremely

rare, and in a review of 532 cases by Bickers et al. [12],

only two cases of broad ligament hydatids were identified.

Most of the ovarian hydatid cysts are seen in elderly and

middle-aged females. The treatment of hydatid disease is

chiefly surgical, and albendazole is given preoperatively to

reduce their size. The overall prognosis of these cysts is

good with reported recurrence of 7–14 % [13]. Recurrence

is chiefly attributed to rupture of the cyst. In cases of

ovarian hydatids, cystectomy is the treatment of choice,

while complete cyst excision with preservation of maxi-

mum possible pulmonary tissue is advocated for lung

hydatids.

Conflict of interest The authors report no conflict of interest.

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Fig. 5 Contrast-enhanced CT

scan of the abdomen showing a

multilocular cystic lesion with a

‘‘cart-wheel’’ appearance in the

right hepatic lobe (white arrow)

and another lesion with

daughter cysts (black arrow)

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